Abstract
Neuromuscular junction (NMJ) disorders include several dysfunctions that ultimately lead to muscle weakness. Myasthenia gravis (MG) is the most prevalent NMJ disorder with a highly polymorphic clinical presentation and many different faces. Being an autoimmune disease, MG correlates with the presence of detectable antibodies directed against the acetylcholine receptor, muscle-specific kinase, lipoprotein-related protein 4, agrin, titin, and ryanodine in the postsynaptic membrane at the NMJ. MG has become a prototype serving to understand both autoimmunity and the function of the NMJ better. The aim of this review is to synthesize some of the epidemiological data available. Epidemiological data regarding MG are important for postulating hypotheses regarding its etiology and facilitating the description of MG subtypes. Thus, adequate documentation through broad databases is essential. The incidence and prevalence of MG reported around the globe have been rising steadily and consistently over the past decades. Ethnic aspects, gender-related differences, and environmental risk factors have been described, implying that these might contribute to a specific phenotype, further suggesting that MG may be considered an umbrella term that covers several clinical entities.
Highlights
Neuromuscular junction (NMJ) disorders comprise several dysfunctions that lead to muscle weakness
Given its pathogenetic mechanism and specific anatomical location, myasthenia gravis (MG) has become both a prototype for autoimmune disorders and a model for understanding the synaptic function [3]. It is an autoimmune B-cell mediated disease and correlates with the presence of antibodies directed against the acetylcholine receptor (AChR), muscle-specific kinase (MuSK), lipoprotein-related protein 4 (LRP4), agrin, titin, and ryanodine in the postsynaptic membrane at the NMJ [3]
The results revealed a higher frequency of ocular MG in the ASH group [47]
Summary
Neuromuscular junction (NMJ) disorders comprise several dysfunctions that lead to muscle weakness. The number of MG patients is growing, and it has more than doubled in the last 20 years This rise is mostly due to a greater MG incidence in the elderly, probably because of the better diagnosis, treatment, and increasing longevity of the population. One of them is the bimodal age distribution with two peaks of incidence: early-onset MG in the third decade (mostly females) and late-onset MG in the elderly (mostly males) [6] Another epidemiological riddle is the ethnic discrepancy observed when comparing cohorts with different racial origins. Depending on the geographic location, the prevalence of MG ranges between 1.5 to 17.9 [1], or between and 2.19 to 36.71 cases/100.000 population (Table 1) This indicates an estimate of 56,000–123,000 patients in Europe [8] and 60.000 in the United States [5]. Neuromuscular Diseases section, the HMO electronic medical records at Hospital Italiano de Buenos Aires
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